The knowledge of clinical features and, particularly, histopathological spectrum of EWSR1-PATZ1-rearranged spindle and round cell sarcomas (EPS) remains limited. For this reason, we report the largest clinicopathological study of EPS to date. Nine cases were collected, consisting of four males and five females ranging in age from 10 to 81 years (average: 49 years). Five tumors occurred in abdominal wall soft tissues, three in the thorax, and one in the back of the neck. Tumor sizes ranged from 2.5 to 18 cm (average 6.6 cm). Five patients had follow-up with an average of 38 months (range: 18-60 months). Two patients had no recurrence or metastasis 19 months after diagnosis. Four patients developed multifocal pleural or pulmonary metastasis and were treated variably by surgery, radiotherapy, and chemotherapy. The latter seemed to have little to no clinical benefit. One of the four patients was free of disease 60 months after diagnosis, two patients were alive with disease at 18 and 60 months, respectively. Morphologically, low, intermediate, and high-grade sarcomas composed of a variable mixture of spindled, ovoid, epithelioid, and round cells were seen. The architectural and stromal features also varied, resulting in a broad morphologic spectrum. Immunohistochemically, the following markers were most consistently expressed: S100-protein (7/9 cases), GFAP (7/8), MyoD1 (8/9), Pax-7 (4/5), desmin (7/9), and AE1/3 (4/9). By next-generation sequencing, all cases revealed EWSR1-PATZ1 gene fusion. In addition, 3/6 cases tested harbored CDKN2A deletion, while CDKN2B deletion and TP53 mutation were detected in one case each. Our findings confirm that EPS is a clinicopathologic entity, albeit with a broad morphologic spectrum. The uneventful outcome in some of our cases indicates that a subset of EPS might follow a more indolent clinical course than previously appreciated. Additional studies are needed to validate whether any morphological and/or molecular attributes have a prognostic impact.

• MeSH
• dítě MeSH
• dospělí MeSH
• fenotyp MeSH
• fúze genů MeSH
• genetická predispozice k nemoci MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádorové biomarkery analýza   genetika   MeSH
• nádory měkkých tkání chemie   genetika   patologie   chirurgie   MeSH
• protein EWS vázající RNA genetika   MeSH
• represorové proteiny genetika   MeSH
• sarkom chemie   genetika   patologie   chirurgie   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• transkripční faktory Krüppel-like genetika   MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• práce podpořená grantem MeSH
• Geografické názvy
• Evropa MeSH
• Spojené státy americké MeSH

BACKGROUND:  Primary cardiac sarcoma (CS) is an extremely rare disease. This study aims to identify possible prognostic factors for long-term survival. METHODS:  A total of 17 consecutive patients who were treated for primary CS between 2003 und 2018 at two cardiac centers were investigated. Clinical data and histological characteristics of the tumors were analyzed. Long-term follow-up of all patients were performed. RESULTS:  The median age was 54 years (range: 23-74). The tumors originated from the left side of the heart in nine patients. Histologically, there were four angiosarcomas, three intimal sarcomas, and three synovial sarcomas. One- and 7-year survivals were 81.9 and 18.2%, respectively. Low expression levels of Ki-67 tended to be associated with increased survival (log-rank p = 0.06). Adjuvant chemotherapy but not radiotherapy regardless of existing metastases was associated with significantly increased survival (log-rank p = 0.001). CONCLUSION:  Angiosarcoma was the most common type of CS. The survival of CS patients is poor but prognostic factors, such as Ki-67, may help estimate the course of the disease. Survival could be improved significantly with chemotherapy.

• MeSH
• adjuvantní chemoterapie MeSH
• antigen Ki-67 analýza   MeSH
• časové faktory MeSH
• dospělí MeSH
• kardiochirurgické výkony * škodlivé účinky   mortalita   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• nádory srdce chemie   mortalita   patologie   chirurgie   MeSH
• přežívající * MeSH
• proliferace buněk MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• sarkom chemie   mortalita   sekundární   chirurgie   MeSH
• senioři MeSH
• výsledek terapie MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Geografické názvy
• Německo MeSH

During the last 30 years many advances have been made in kidney tumor pathology. In 1981, 9 entities were recognized in the WHO Classification. In the latest classification of 2004, 50 different types have been recognized. Additional tumor entities have been described since and a wide variety of prognostic parameters have been investigated with variable success; however, much attention has centered upon the importance of features relating to both stage and grade. The International Society of Urological Pathology (ISUP) recommends after consensus conferences the development of reporting guidelines, which have been adopted worldwide ISUP undertook to review all aspects of the pathology of adult renal malignancy through an international consensus conference to be held in 2012. As in the past, participation in this consensus conference was restricted to acknowledged experts in the field.

• MeSH
• cystická onemocnění ledvin patologie   MeSH
• dědičné nádorové syndromy patologie   MeSH
• diferenciální diagnóza MeSH
• dospělí MeSH
• karcinom chemie   klasifikace   diagnóza   genetika   patologie   MeSH
• leiomyom genetika   patologie   MeSH
• lidé MeSH
• metastázy nádorů MeSH
• nádorové biomarkery analýza   MeSH
• nádorové proteiny analýza   genetika   MeSH
• nádory ledvin chemie   klasifikace   diagnóza   genetika   patologie   MeSH
• prognóza MeSH
• sarkom chemie   patologie   MeSH
• stupeň nádoru MeSH
• translokace genetická MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• konsensus - konference MeSH
• směrnice pro lékařskou praxi MeSH

Soft tissue sarcomas (STSs) are rare heterogeneous tumors with variable clinical course and outcome. The management of STSs depends upon the accurate histopathological diagnosis and assessing their histological grade. Currently, core needle biopsies are becoming increasingly popular for diagnosing STSs but value of histological grading is limited from this type of specimens. To evaluate the immunohistochemical expression of p53, mdm-2, cyclin D1, p16, nm23, EGFR and Ki-67 labelling index in adult STSs patients and their association with histological grade of STSs, we analysed 101 primary untreated STSs of the limbs and trunk using the tissue microarray technique on formalin-fixed, paraffin-embedded tissue samples. The cases consisted of 15 G1, 28 G2 and 58 G2 sarcomas. Ki-67 labelling index (LI) was calculated from whole block sections for the possibility to select the most proliferative regions. The LI ranged from 1.26 to 75.5% (median 26.7%) and strongly correlated with the mitotic count (p rs for adult patients with STSs and may assist in establishment of the histological grade in STSs.

• MeSH
• čipová analýza tkání metody   MeSH
• cyklin D1 analýza   MeSH
• dospělí MeSH
• erbB receptory analýza   MeSH
• imunohistochemie MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• nádorový supresorový protein p53 analýza   MeSH
• NM23-nukleosiddifosfátkinasy analýza   MeSH
• sarkom chemie   patologie   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH

The aim of our study was to examine in vivo and in vitro cytokines produced by Lewis ratderived R5-28 sarcoma cells. These cells produce rapidly growing tumours in approximately two weeks after subcutaneous inoculation. However, spontaneous tumour regression was noted in about 40% of animals. For an explanation of this phenomenon, we evaluated the profile of 19 cytokines during tumour growth and spontaneous regression by the use of "antibody array". To detect cytokines directly originated by the sarcoma, the R5-28 cells were cultivated in vitro and then both the supernatants and the cell lysates were analysed. Our experiments showed three cytokines (MCP-1, TIMP-1 and VEGF) to be produced by R5-28 cells in vitro. Moreover, in vivo, another three cytokines (TNF-alpha, beta-NGF and LIX) were detected both in blood sera and tumour lysates, probably produced by immune and stromal cells during tumour growth. Changes in their expression after spontaneous regression are discussed.

• MeSH
• cytokininy chemie   MeSH
• financování organizované využití   MeSH
• interleukiny chemie   MeSH
• invazivní růst nádoru imunologie   MeSH
• modely nemocí na zvířatech MeSH
• nádorové buňky kultivované MeSH
• potkani inbrední LEW MeSH
• sarkom chemie   MeSH
• spontánní regrese nádoru imunologie   MeSH

• MeSH
• antigeny diferenciační myelomonocytární analýza   MeSH
• buněčný cyklus MeSH
• CD antigeny analýza   MeSH
• diferenciální diagnóza MeSH
• dítě MeSH
• DNA nádorová analýza   MeSH
• lidé MeSH
• mladiství MeSH
• předškolní dítě MeSH
• sarkom chemie   patologie   ultrastruktura   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH